Study On Clinical Characteristics And Multimodal Imaging Of Autoimmune Retinopathy

Objective: To investigate and analyze clinical characteristics and multimodal imaging of patients with autoimmune retinopathy(AIR).Methods: Three patients(6 eyes)with AIR confirmed by the Second Hospital of Hebei Medical University from December 2017 to December 2019 were included in the study.All patients underwent comprehensive ophthalmological examinations,including Vision acuity,intraocular pressure(IOP),slit lamp and indirect ophthalmoscopy examinations.In terms of imaging,Spectral-domain optical coherence tomography(SD-OCT),fundus autofluorescence(FAF),and fundus fluorescein angiography(FFA)examinations were performed.Patients were also tested for full-field electroretinogram(ff ERG),visual field examination,and explore the presence of serum anti-retinal antibodies(ARA).Results: 1.Among the 3 patients with AIR,1 was cancer-associated retinopathy(CAR),and 2 were non-paraneoplastic autoimmune retinopathy(np-AIR).The youngest patient is 48 years old and the oldest is 54 years old,both of which are female;2.Clinical characteristics,3 cases had subacute and progressive decreasing visual acuity,the lesion of both eyes is developed simultaneously or sequentially,2 cases with photopsia,1 case with color deficiencies.the degree of vision loss was moderate,and the best corrected visual acuity(BCVA)was Between 0.1 to 0.15.There were no obvious abnormalities in the anterior segment and fundus examination;3.Multi-mode image characteristics observation,SD-OCT examination found that 3 patients Ellipsoid Zone(EZ)outside foveomacular region was disruption or thinning and lost,the outer nuclear layer(ONL)was thinning to varying degrees,and the inner retina was roughly normal.The choroidal capillary layer become atrophic.the retinal structure in foveomacular region was relatively complete,and a "flying sau-cer" sign similar to that of Hydroxychlor-oquine retinopathy can be seen in foveomacular region.FAF examination revealed that two patients presented with a parafoveal hyperautofluorescent ring.SD-OCT examination of the hyperautofluorescent ring area found that the hyperautofluorescent ring area was corresponds to the loss of the outer layer structure of the retina.Inside the ring,all retinal layers were observed,but there was interrupted or thinning of the Ellipsoid Zone and ONL as they approached the inner border of the ring;across the ring,there was obvious thinning of Ellipsoid Zone and ONL;Outside the hyperautofluorescent ring,there was further thinner of Ellipsoid Zone and ONL.FFA examination revealed that 2 patients presented with change to retinalvenous vasculitis,in venous phase,The main and peripheral retinal vein with segmental tube wall staining and fluorescein leakage in late venous phase;4.Other ophthalmological examinations: Visual field examination of 3 patients showed arcuate or annular visual field defects.ff ERG shows moderate to severe amplitude reductions in light adaptation and dark adaptation in a and b waves,with prolonged peak times.3 patients received serum anti-retinal antibody(ARA)tests,all of which were positive,of which 3 were positive for α-enolase antibody,3 were positive for Carbonic Anhydrase II antibody,and 3 were positive for CRMP-5 antibody;5.The therapy and follow-up results,Among them,2 patients received glucocorticoid and immunosuppressor therapy.At the end of follow-up,the vision of 4 eyes remained stable and the visual field improved.Another patient was treated with traditional Chinese medicine on his own and was loss to follow-up after 4 monthsConclusions:1.Acute or subacute vision loss,ERG abnormality,and serum retinal antibody positive are the common characteristics of AIR patients.2.Disruption or thinning and loss of Ellipsoid Zone(EZ)outside foveomacular region,thinning of outer nuclear layer(ONL)and hyperautofluorescent ring of FAF are more characteristic manifestations of AIR.3.Multimodal imaging examination Combined with SD-OCT,FAF and FFA,provides objective evidence for AIR retinal damage,and provides clues for the diagnosis of AIR and understanding the mechanism of retinal dysfunction.